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Idiopathic pulmonary hemosiderosis in a 9-year-old girl

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Author(s): Kamienska E | Urasinski T | Gawlikowska-Sroka A | Glura B | Pogorzelski A

Journal: European Journal of Medical Research
ISSN 2047-783X

Volume: 14;
Issue: Suppl 4;
Start page: 112;
Date: 2009;
Original page

Keywords: diffuse alveolar | hemoptysis | hemorrhage | hemosiderosis | idiopathic pulmonary

ABSTRACT
Abstract Diffuse alveolar hemorrhage (DAH) is a rare and life-threatening condition characterized by hemoptysis, dyspnoea, alveolar infiltrates on chest radiograph and various degrees of anemia. It may occur either as a primary disease of the lungs or a secondary condition due to cardiac, systemic vascular, collagen or renal diseases. Idiopathic pulmonary hemosiderosis (IPH) is a separate form of DAH of unknown origin, associated in some cases with celiac disease. The estimated incidence of IPH in children is 0.24-1.23 cases per million, with a mortality rate as high as 50%. Only about 500 cases of this disease have been described in medical literature. We present a case of a 9-year-old girl diagnosed with IPH, which was confirmed by the presence of many hemosiderin-laden macrophages in bronchoalveolar lavage obtained by bronchofiberoscopy. Therapy with glucocorticoids was initiated with a partial and transient response. Azathioprine and a gluten-free diet were subsequently introduced. However, the girl still suffers from recurrent episodes of hemoptysis, dyspnea and anemia.
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